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Table of contents

Achalasia

Background

Overview

Definition
Achalasia is an esophageal motility disorder defined by loss of esophageal peristalsis and incomplete relaxation of lower esophageal sphincter.
1
Pathophysiology
Proposed causes of achalasia include GEJ obstruction, neuronal degeneration, viral infection, genetic inheritance, and autoimmune disease.
2
Disease course
Inflammatory neurodegenerative insult with possible viral involvement within the esophagus results in achalasia, which causes the clinical manifestation of progressive dysphagia, regurgitation, chest pain, heartburn, nocturnal cough, aspiration, and weight loss. The progression of the disease may result in aspiration-pneumonia, Barrett's esophagus, and esophageal cancer.
3
4
Prognosis and risk of recurrence
Achalasia is not associated with an increase in mortality.
5

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of achalasia are prepared by our editorial team based on guidelines from the American Society for Gastrointestinal Endoscopy (ASGE 2020,2014), the European Society of Gastrointestinal Endoscopy (ESGE 2020), the International Society for Diseases of the Esophagus (ISDE 2018), and the American College of Gastroenterology (ACG 2013). ...
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Screening and diagnosis

Diagnosis: as per ACG 2013 guidelines, suspect achalasia in patients with dysphagia to solids and liquids, and in patients with regurgitation unresponsive to an adequate trial of PPI therapy.
B
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Classification and risk stratification

Disease classification: as per ISDE 2018 guidelines, use the Chicago classification of esophageal motility disorders to assign patients with achalasia to one of three disease subclasses:
Situation
Guidance
Type I (classic)
Elevated median integrated relaxation pressure (> 15 mmHg)
100% failed peristalsis
Distal contractile integral < 100 mmHg
Premature contractions with distal contractile integral < 450 mmHg cm/sec satisfy criteria for failed peristalsis
Type II (with esophageal compression)
Elevated median integrated relaxation pressure (> 15 mmHg)
100% failed peristalsis
Panesophageal pressurization with ≥ 20% of swallows
Contractions may be masked by esophageal pressurization and distal contractile integral should not be calculated
Type III (spastic)
Elevated median integrated relaxation pressure (> 15 mmHg)
No normal peristalsis
Premature (spastic) contractions with distal contractile integral > 450 mmHg cm/sec with ≥ 20% of swallows
May be mixed with panesophageal pressurization
E

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  • Severity grading

Diagnostic investigations

Esophageal manometry: as per ISDE 2018 guidelines, consider obtaining high-resolution esophageal manometry as first-line investigation for the diagnosis of achalasia.
C

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  • Barium esophagram

  • Upper gastrointestinal endoscopy

Medical management

Pharmacologic therapy: as per ISDE 2018 guidelines, avoid using nitrates, calcium blockers or phosphodiesterase inhibitors for symptomatic relief in patients with achalasia.
D

Therapeutic procedures

Choice of initial therapy: as per ASGE 2020 guidelines, offer laparoscopic Heller myotomy, pneumatic dilation, and POEM as effective therapeutic modalities in patients with achalasia. Decide between these treatment options based on achalasia type, local expertise, and patient preference.
A
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  • Pneumatic dilatation

  • POEM

  • Botulinum toxin injection

  • Other endoscopic therapies

Perioperative care

Perioperative antibiotic prophylaxis: as per ESGE 2020 guidelines, administer perioperative antibiotic prophylaxis for POEM. Adapt the choice and duration of antibiotics according to national or local protocols.
B

Surgical interventions

Surgical myotomy: as per ISDE 2018 guidelines, perform laparoscopic Heller myotomy for symptom control in patients with Chicago type I and type II achalasia.
B
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  • Esophagectomy

Specific circumstances

Pediatric patients: as per ISDE 2018 guidelines, obtain the same work-up in pediatric patients with a provisional diagnosis of achalasia as in adult patients.
E
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  • Patients with Chagas disease esophagopathy

  • Patients with other spastic motility disorders

Patient education

General counseling: as per ISDE 2018 guidelines, inform male patients with achalasia about the moderately increased risk of esophageal cancer after ≥ 10 years from the initial treatment.
E

Follow-up and surveillance

Post-treatment follow-up
As per ISDE 2018 guidelines:
Assess patients for symptomatic improvement after treatment for achalasia.
E
Consider obtaining a timed barium esophagram to assess for recurrence of achalasia.
C

More topics in this section

  • Management of disease recurrence

  • Surveillance endoscopy